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Three major GTP-binding protein families include trimeric and low molecular weight G-proteins, as well as a family of large proteins homologous to dynamin. The dynamin family contains proteins with diverse structure and function, but highly homologous N-terminal GTPase domains. A subgroup of the dynamin G-protein-binding family includes the mitochondrial proteins Drp1/Dnm1, Mgm1, and OPA1. The latter protein is mutated in dominant optic atrophy, a disease that involves loss of visual acuity and atrophy of the optic nerve. OPA1 is expressed in heart, brain, liver, and kidney. The sequence of OPA1 includes an N-terminal region that contains a mitochondrial targeting domain and three GTP-binding motifs. The overexpression of OPA1 in Cos-7 cells shows co-localization with cytochrome c in mitochondria, and leads to alterations in mitochondrial morphology from a characteristic tubuluar shape to a vesicular pattern. Thus, OPA1 may have roles in mitochondrial biogenesis that are critical for normal cell function. This antibody is routinely tested by western blot analysis. Other applications were tested at BD Biosciences Pharmingen during antibody development only or reported in the literature.Immunofluorescence, Western Blotting
